期刊
JOURNAL OF AUTOIMMUNITY
卷 34, 期 1, 页码 66-72出版社
ACADEMIC PRESS LTD- ELSEVIER SCIENCE LTD
DOI: 10.1016/j.jaut.2009.07.002
关键词
Autoimmunity; Tolerance; Autoimmune polyendocrine; syndrome type I; Autoimmune regulator gene; iNKT cells; NK cells
类别
资金
- Swedish Medical Council
- Swedish Research Council
- Lundberg foundation
- Ronald McDonald foundation
- Gronwalls foundation
- Magnus Bergvall foundation
- Sven Jerring Foundation
- Swedish Diabetes Association
- Swedish Medical Society
- Agnes and Mac Rudbergs foundation
- Swedish Cancer Society
- Wenner-Gren Foundations
- Karolinska Institutet
Autoimmune Polyendocrine Syndrome type I (APS I) is caused by mutations in the Autoimmune Regulator gene (AIRE), and results in the immunological destruction of endocrine organs. Herein we have characterized the CD1d-restricted invariant NKT cells (iNKT) and NK cells in APS I patients and Aire(-/-) mice, two cell populations known to play a role in the regulation of autoimmune disease. We show that the frequency of circulating iNKT cells is reduced in APS I patients compared to healthy controls. In accordance with this, iNKT cells are significantly reduced in the thymus and peripheral organs of Aire(-/-) mice. Bone marrow transfer from wild type donors into lethally irradiated Aire(-/-) recipients led to a decreased iNKT cell population in the liver, suggesting an impaired development of iNKT cells in the absence of Aire expression in radio-resistant cells. In contrast to the iNKT cells, both conventional NK cells and thymus-derived NK cells were unaffected by Aire deficiency and differentiated normally in Aire(-/-) mice. Our results show that expression of Aire in radio-resistant cells is important for the development of iNKT cells, whereas NK cell development and function does not depend on Aire. (C) 2009 Elsevier Ltd. All rights reserved.
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